미로누공을 동반한 선천성진주종 1례

Congenital cholesteatoma of the middle ear is now being reported increasingly in otologic literature. Derlacki’s classic description of congenital cholesteatoma is that of a keratotic mass behind an intact tympanic membrane with no history of otitis media, otorrhea, or otological surgical procedure. The most important criterion for diagnosis is the presence of an intact tympanic membrane. We recently experienced a case of congenital cholesteatoma with large labyrinthine fistula in 41 year old male patient who showed normal tympanic membrane without prior history of ear disease. Cholesteatoma was localized within the antrum of mastoid. Destruction of the lateral semicircular canal was observed. Complete surgical removal·was performed using an canal wall up mastoidectomy. There is no significant difference between preoperative and postoperative bone conduction threshold, 5 dB and 13 dB respectively.

We report this case with brief review of the literatures.